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CineECG analysis provides new insights into Familial ST-segment Depression Syndrome

AIMS: Familial ST-segment Depression Syndrome (Fam-STD) is a novel inherited cardiac disease associated with arrhythmias and sudden cardiac death. This study aimed at investigating the cardiac activation pathway in patients with Fam-STD, modelling the electrocardiogram (ECG) phenotype, and performin...

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Autores principales: Frosted, Rasmus, Paludan-Müller, Christian, Vad, Oliver Bundgaard, Olesen, Morten Salling, Bundgaard, Henning, van Dam, Peter, Christensen, Alex Hørby
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10228618/
https://www.ncbi.nlm.nih.gov/pubmed/37140072
http://dx.doi.org/10.1093/europace/euad116
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author Frosted, Rasmus
Paludan-Müller, Christian
Vad, Oliver Bundgaard
Olesen, Morten Salling
Bundgaard, Henning
van Dam, Peter
Christensen, Alex Hørby
author_facet Frosted, Rasmus
Paludan-Müller, Christian
Vad, Oliver Bundgaard
Olesen, Morten Salling
Bundgaard, Henning
van Dam, Peter
Christensen, Alex Hørby
author_sort Frosted, Rasmus
collection PubMed
description AIMS: Familial ST-segment Depression Syndrome (Fam-STD) is a novel inherited cardiac disease associated with arrhythmias and sudden cardiac death. This study aimed at investigating the cardiac activation pathway in patients with Fam-STD, modelling the electrocardiogram (ECG) phenotype, and performing in-depth ST-segment analyses. METHODS AND RESULTS: CineECG analysis of patients with Fam-STD and age- and sex-matched controls. The groups were compared using the CineECG software which included the trans-cardiac ratio and the electrical activation pathway. We simulated the Fam-STD ECG phenotype by adjusting action potential duration (APD) and action potential amplitude (APA) in specific cardiac regions. High-resolution ST-segment analyses were performed per lead by dividing the ST-segment into nine 10 ms subintervals. Twenty-seven Fam-STD patients (74% females, mean age 51.6 ± 6.2 years) and 83 matched controls were included. Among Fam-STD patients, electrical activation pathway analysis in the anterior-basal orientation showed significantly abnormal direction toward the basal areas of the heart starting from QRS 60–89 ms until Tpeak-Tend (all P < 0.001). Simulations with shortened APD and reduced APA in the left ventricle basal regions recapitulated the Fam-STD ECG phenotype. Detailed ST-segment analyses showed significant differences in all nine 10 ms subintervals (all P < 0.01), with the most prominent findings during the 70–79/80–89 ms intervals. CONCLUSION: CineECG analyses indicated abnormal repolarization with basal directions, and the Fam-STD ECG phenotype was simulated by reducing APD and APA in the left ventricle basal regions. Detailed ST-analysis showed amplitudes consistent with the proposed diagnostic criteria for Fam-STD patients. Our findings provide new insight into the electrophysiological abnormalities of Fam-STD.
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spelling pubmed-102286182023-05-31 CineECG analysis provides new insights into Familial ST-segment Depression Syndrome Frosted, Rasmus Paludan-Müller, Christian Vad, Oliver Bundgaard Olesen, Morten Salling Bundgaard, Henning van Dam, Peter Christensen, Alex Hørby Europace Translational Research AIMS: Familial ST-segment Depression Syndrome (Fam-STD) is a novel inherited cardiac disease associated with arrhythmias and sudden cardiac death. This study aimed at investigating the cardiac activation pathway in patients with Fam-STD, modelling the electrocardiogram (ECG) phenotype, and performing in-depth ST-segment analyses. METHODS AND RESULTS: CineECG analysis of patients with Fam-STD and age- and sex-matched controls. The groups were compared using the CineECG software which included the trans-cardiac ratio and the electrical activation pathway. We simulated the Fam-STD ECG phenotype by adjusting action potential duration (APD) and action potential amplitude (APA) in specific cardiac regions. High-resolution ST-segment analyses were performed per lead by dividing the ST-segment into nine 10 ms subintervals. Twenty-seven Fam-STD patients (74% females, mean age 51.6 ± 6.2 years) and 83 matched controls were included. Among Fam-STD patients, electrical activation pathway analysis in the anterior-basal orientation showed significantly abnormal direction toward the basal areas of the heart starting from QRS 60–89 ms until Tpeak-Tend (all P < 0.001). Simulations with shortened APD and reduced APA in the left ventricle basal regions recapitulated the Fam-STD ECG phenotype. Detailed ST-segment analyses showed significant differences in all nine 10 ms subintervals (all P < 0.01), with the most prominent findings during the 70–79/80–89 ms intervals. CONCLUSION: CineECG analyses indicated abnormal repolarization with basal directions, and the Fam-STD ECG phenotype was simulated by reducing APD and APA in the left ventricle basal regions. Detailed ST-analysis showed amplitudes consistent with the proposed diagnostic criteria for Fam-STD patients. Our findings provide new insight into the electrophysiological abnormalities of Fam-STD. Oxford University Press 2023-05-04 /pmc/articles/PMC10228618/ /pubmed/37140072 http://dx.doi.org/10.1093/europace/euad116 Text en © The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology. https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Translational Research
Frosted, Rasmus
Paludan-Müller, Christian
Vad, Oliver Bundgaard
Olesen, Morten Salling
Bundgaard, Henning
van Dam, Peter
Christensen, Alex Hørby
CineECG analysis provides new insights into Familial ST-segment Depression Syndrome
title CineECG analysis provides new insights into Familial ST-segment Depression Syndrome
title_full CineECG analysis provides new insights into Familial ST-segment Depression Syndrome
title_fullStr CineECG analysis provides new insights into Familial ST-segment Depression Syndrome
title_full_unstemmed CineECG analysis provides new insights into Familial ST-segment Depression Syndrome
title_short CineECG analysis provides new insights into Familial ST-segment Depression Syndrome
title_sort cineecg analysis provides new insights into familial st-segment depression syndrome
topic Translational Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10228618/
https://www.ncbi.nlm.nih.gov/pubmed/37140072
http://dx.doi.org/10.1093/europace/euad116
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